Purpose To investigate the clinicopathologic features of calcifying aponeurotic fibroma (CAF). Methods The clinical and pathologic profiles of 6 cases of CAF were retrospectively analyzed. Results CAF appeared a firm, painless and slowly growing mass with a predilection for the distal portions of the extremities in young children and adolescents. Microscopically, the tumor consis-ted mainly of benign appearing spindle cells, chondroid cells and calcific debris. Immunohistochemically, the tumor cells were positive for vimentin, HHF-35, S-100, Calponin and CD68. Conclusion CAF is a rare, locally aggressive fibroblastic lesion. It can make an accurate diagnosis by integrating the clinical location with histopathological features. Repeated minor trauma to the extremities and some embryologic cell nests maybe its pathogenesis.

OBJECTIVE:To establish a method for the content determination of AV45(the labeled precursor of PET agent for Aβ plaques)and its related substances. METHODS:HPLC method was performed on the column of Inertsil C18 with mobile phase of 50 mmol/L Disodium hydrogen phosphate solution- acetonitrile(containing 0.01% triethylamine)(15∶85,V/V)at a flow rate of 1.0 ml/min,the detection wavelength was 325 nm,and column temperature was 30 ℃,and volume injection was 20 μl. RESULTS:The AV45 and reaction intermediate as well as individual impurity peaks were well separated;the linear range was 200-1 000 μg/ml for AV45(r=0.999 2)and 20-100 μg/ml for intermediate(r=0.999 6);the detection limit and quantification limit of reaction in-termediate AV45 were 0.1 ng and 0.5 ng,respectively;RSDs of precision,stability and reproducibility tests were lower than 0.5%;recovery of AV45 was 98.93%-101.58%(RSD=0.69%,n=9). CONCLUSIONS:The method is simple and accurate with high specificity and good reproducibility,and suitable for content determination of AV45(the labeled precursor of PET agent for Aβplaques)and its related substances.

Purpose To investigate the clinicopathologic features of desmoplastic fibroma of bone (DF).Methods The clinical and pathologic profiles of 7 cases were retrospectively analyzed.Results Desmoplastic fibroma of bone was a rare neoplasm of bone,which typically occurred in young children and adolescents.The tumor showed infiltrative and locally aggressive nature,the most common sites were long tubular bones and mandible.Microscopically,the tumor was composed of sparse fibroblasts in a rich background of collagen fibers.Immunohistochemically,the tumor cells were positive for vimentin and SMA.Conclusion Desmoplastic fibroma is a rare,locally aggressive fibroblasticlesion of bone.It can make an accurate diagnosis by integrating the clinical location with histopathological features.Trauma,hormone and horomosome aberration may be related with its pathogenesis.

A 22-year-old patient was admitted to the hospital with a solitary,gradually growing and painless mass in the left shoulder for 2 years.Physical examination revealed no abnormality except for the skin lesion.Skin examination showed an elevated lesion measuring about 3.5 cm× 2.0 cm × 1.5 cm with smooth surface and normal color,which was located in the subcutaeous tissue,indurated and movable.Resection of the tumor was performed under local anesthesia.On visual observation during operation,the tumor was sited in the subcutaneous fat tissue,nodular-like and surrounded locally by fibrous pseudocapsules with a grey incisal surface and mild texture.Microscopicalty,the tumor was extremely similar to breast fibroadenoma with multiple lobuli,and each of the lobuli was composed of tubiform structures,basal cell-like epithelial cell trabs and fibromyxoid stroma abundant in fibroblast-like spindle cells.No hair bulb or primitive dermal papillae were observed in the lobuli,which were separated by compact collagen fibers infiltrated by a few scattered inflammatory cells.Fibromyxoid strotma was surrounded by basal cell-like epithelial cell strabs in most lobuli,and some tubiform structures were filled with a little thin lightly eosinophilic material in a concentric arrangement.Immunohistochemistry showed that intralobular epithelial cells were strongly positive for cytokeratin 5/6,but negative for CAM5.2 or carcinoembryonic antigen (CEA).In addition,the lightly eosinophilic material in lumens was negative for periodic acid-Schiff (PAS) staining.These results suggested that the tubiform structures were immature follicles,but not sweat ducts.The patient was diagnosed with nodular fibrofolliculoma (NFF) based on the clinical manifestations,morphological features,immunohistochemical and PAS staining results.No relapse was observed in more than 3 months of postoperative follow-up.As a benign trichogenic adnexal neoplasm with unique clinicopathological manifestations,NFF may be a new entity of cutaneous adnexal neoplasm.

Moyamoya disease is a chronic progressive cerebrovascular disease. Its disability rate and lethality rate are higher. The direct and indirect revascularization can increase cerebral blood flow and reduce the occurrence of cerebral ischemia and cerebral hemorrhage. This article reviews the pathophysiological basis of its surgical treatment, surgical timing, indications, surgical treatment methods and efficacy.

A 13-year-old female patient presented with itchy rashes on the right foot for 5 years. Skin examination revealed scattered flat, reddish-purple, slightly scaling papules on the inner side of the arch of the right foot, scattered light red papules on the outer side of the right great toe, and scattered domed reddish-purple papules on the right heel with collar-shaped keratinization around the base. Histopathological examination of the papule on the right heel revealed epidermal hyperkeratosis, marked hyperkeratosis around the papule, thickened granular layer and focal liquefaction degeneration of the basal layer with no obvious hyperplasia of the spinous layer; a large number of lymphocytes and plasma cells without atypia infiltrated the dermis, in which there were many small- and medium-sized dilated thick-walled blood vessels lined with prominent plump endothelial cells. The patient was diagnosed with acral pseudolymphomatous angiokeratoma of children, and treated with long-pulsed 1 064-nm Nd: YAG laser once every month. After 7 sessions of treatment, the rashes regressed and decreased in number.

Objective:To investigate the clinicopathological and genetic features of epithelioid and spindle cell rhabdomysarcoma with EWSR1-TFCP2 or FUS-TFCP2 fusion.Methods:The clinical, morphological and immunohistochemical features of 14 cases of epithelioid and spindle cell rhabdomysarcoma with EWSR1-TFCP2 or FUS-TFCP2 fusion diagnosed from January 2019 to December 2022 in the Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed. The cases were all subject to FISH or next generation sequencing for analysis of molecular genetic features. The literature was reviewed.Results:There were 5 males and 9 females, with the age at presentation ranging from 6 to 36 years (mean, 22 years). Tumors occurred in the head and neck (9 cases), pelvic region (2 cases), bladder (one case), right humerus (one case), and the abdominal wall, humerus and pubic at the same time (one case). Presenting symptoms varied by location but often included pain or discomfort. Most of the patients showed aggressive radiographic features with soft tissue extension. The tumors had a median size of 6.6 cm (range, 2-23 cm). The tumors were poorly defined and irregularly shaped. Microscopic examination showed diffuse proliferation of spindle or epithelioid cells. While morphologically high-grade tumors displayed obvious cytological atypia, a high mitotic count and tumor necrosis, low-grade tumors grew in sheets and fascicles composed of spindle, epithelioid cells with moderate or abundant amounts of eosinophilic cytoplasm, without pronounced cytological atypia. The tumor cells expressed Desmin, MyoD1, and Myogenin, as well as ALK, EMA, and CKpan. EWSR1/FUS-TFCP2 gene fusion was detected in 14 cases with next generation sequencing and confirmed by FISH. Six cases had EWSR1-TFCP2 fusions and 8 cases showed FUS-TFCP2 fusions. Follow-up information was available in 13 patients, ranged from 5 to 37 months. At the end of follow-up period, 7 patients died of the disease. Six patients were alive:two cases had local recurrences and metastases, two cases of recurrences, one case of metastasis and one case without recurrences and metastasis.Conclusions:Epithelioid and spindle cell rhabdomysarcomas with EWSR1-TFCP2 or FUS-TFCP2 fusion show a very aggressive clinical course, and more commonly occur in the head and neck. Their genetic hallmark is the presence of EWSR1/FUS-TFCP2 fusions. Familiarity with its clinicopathological characteristics is helpful in avoiding misdiagnoses.

A 25-year-old female patient presented with itchy rashes on her feet for 10 years. Skin examination showed dark red plaques with clear boundaries, whose surfaces were covered with grey-brown scales, on the dorsum of toes and adjacent dorsum of the feet, the extensor aspect of the right ankle and the left Achilles tendon. Laboratory tests revealed that serum anti-hepatitis C virus (HCV) antibody was negative and HCV-RNA quantity was normal. Histopathological examination showed epidermal hyperkeratosis with parakeratosis, irregularly thickened spinous layer, scattered dyskeratotic cells and necrotic keratinocytes in the middle and upper spinous layer, focal liquefaction degeneration of basal cells, dilatation of small blood vessels in the superficial and middle dermis, and perivascular infiltration of a small number of lymphocytes. The patient was diagnosed with necrolytic acral erythema without hepatitis C. After 2-month treatment, the skin lesions completely regressed, and hyperpigmentation remained.